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Clinical and Diagnostic Laboratory Immunology, March 1999, p. 161-167, Vol. 6, No. 2
Department of
Pediatrics,1 Microbiology and
Immunology,2
Pathology,3 and Human Biological
Chemistry and Genetics4 of the University of
Texas Medical Branch, Galveston, Texas
Received 15 July 1998/Returned for modification 27 August
1998/Accepted 7 October 1998
A unique immune deficiency in a 24-month-old male characterized by
a transient but protracted developmental delay in the B-cell lineage is
reported. Significant deficiencies in the number of B cells in the
blood, the concentrations of immunoglobulins in the serum, and the
titers of antibodies to T-dependent and T-independent antigens resolved
spontaneously by the age of 39 months in a sequence that duplicated the
normal development of the B-cell lineage: blood B cells followed by
immunoglobulin M (IgM), IgG, IgA, and specific IgG antibodies to
T-independent antigens (pneumococcal polysaccharides). Because of the
sequence of recovery, the disorder could have been confused with other
defects in humoral immunity, depending on when in the course of disease
immunologic studies were conducted. Investigations of X-chromosome
polymorphisms suggested that the disorder was not X linked in that the
mother appeared to have identical X chromosomes. An autosomal recessive
disorder involving a gene that controls B-cell development and
maturation seems more likely. In summary, this case appears to be a
novel protracted delay in the development of the B-cell lineage,
possibly due to an autosomal recessive genetic defect.
1071-412X/99/$04.00+0
Copyright © 1999, American Society for Microbiology. All rights reserved.
Immunodeficiency Due to a Unique Protracted
Developmental Delay in the B-Cell Lineage
*
Corresponding author. Mailing address: Pediatric
Immunology/Allergy/Rheumatology Division, Room 2.360 Children's
Hospital, The University of Texas Medical Branch, 301 University Blvd., Galveston, TX 77555-0369. Phone: (409) 772-2658. Fax: (409) 747-6622. E-mail: agoldman{at}utmb.edu.
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